Usher Syndrome in Children

What is Usher syndrome in children?

Usher syndrome is a disorder that is passed down through families (inherited). A syndrome is a group of symptoms that happen together. Usher syndrome involves both hearing loss and vision loss. The hearing loss may be mild to complete. The vision problem is called retinitis pigmentosa. The retinas of the eyes are slowly damaged over time. The retinas receive light and play a very important part in being able to see. Usher syndrome is the most common childhood condition that affects both vision and hearing.

What causes Usher syndrome in a child?

Usher syndrome is passed on from parents to their children. It may be inherited when both parents are carriers of an abnormal gene. If both parents have the gene, they have a 1 in 4 chance of having a child with Usher syndrome with each pregnancy.

What are the symptoms of Usher syndrome in a child?

Symptoms depend on the type of Usher syndrome. There are several types and many sub-types of the syndrome. Symptoms of the syndrome may include:

Problems with hearing or deafness

Problems with balance

Problems with eyesight or blindness

The problems with eyesight are caused by an eye disorder called retinitis pigmentosa. This disorder includes a group of eye diseases that leads to gradual loss of eyesight.

How is Usher syndrome diagnosed in a child?

All newborn babies are screened for hearing problems. If a hearing problem is found in the newborn, the baby will have follow-up testing. Your child's healthcare provider will test hearing, eyesight, and balance to diagnose Usher syndrome. The tests include: